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MGP002211

Record overview

MGPD IDMGP002211
Gene ID5096
SpeciesHomo sapiens (Human)
Gene Namepropionyl CoA carboxylase, beta polypeptide
Gene Symbol PCCB
Alternate namespropionyl-CoA carboxylase beta chain, mitochondrial; PCCase subunit beta; propanoyl-CoA:carbon dioxide ligase subunit beta; propionyl Coenzyme A carboxylase, beta polypeptide;
Chromosome3
Map Location3q21-q22
EC Number6.4.1.3
SummaryThe protein encoded by this gene is a subunit of the propionyl-CoA carboxylase (PCC) enzyme, which is involved in the catabolism of propionyl-CoA. PCC is a mitochondrial enzyme that probably acts as a dodecamer of six alpha subunits and six beta subunits. This gene encodes the beta subunit of PCC. Defects in this gene are a cause of propionic acidemia type II (PA-2). Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, May 2010]
OrthologsView orthologs and multiple alignments for PCCB

Proteins

propionyl-CoA carboxylase beta chain, mitochondrial isoform 1 precursor
Refseq ID:NP_000523
Protein GI:119943100
UniProt ID:P05166
mRNA ID:NM_000532
Length:539
RefSeq Status:
MAAALRVAAVGARLSVLASGLRAAVRSLCSQATSVNERIENKRRTALLGGGQRRIDAQHKRGKLTARERISLLLDPGSFVESDMFVEHRCADFGMAADKN
KFPGDSVVTGRGRINGRLVYVFSQDFTVFGGSLSGAHAQKICKIMDQAITVGAPVIGLNDSGGARIQEGVESLAGYADIFLRNVTASGVIPQISLIMGPC
AGGAVYSPALTDFTFMVKDTSYLFITGPDVVKSVTNEDVTQEELGGAKTHTTMSGVAHRAFENDVDALCNLRDFFNYLPLSSQDPAPVRECHDPSDRLVP
ELDTIVPLESTKAYNMVDIIHSVVDEREFFEIMPNYAKNIIVGFARMNGRTVGIVGNQPKVASGCLDINSSVKGARFVRFCDAFNIPLITFVDVPGFLPG
TAQEYGGIIRHGAKLLYAFAEATVPKVTVITRKAYGGAYDVMSSKHLCGDTNYAWPTAEIAVMGAKGAVEIIFKGHENVEAAQAEYIEKFANPFPAAVRG
FVDDIIQPSSTRARICCDLDVLASKKVQRPWRKHANIPL
 
propionyl-CoA carboxylase beta chain, mitochondrial isoform 2 precursor
Refseq ID:NP_001171485
Protein GI:295821216
UniProt ID:P05166
mRNA ID:NM_001178014
Length:559
RefSeq Status:
MAAALRVAAVGARLSVLASGLRAAVRSLCSQATSVNERIENKRRTALLGGGQRRIDAQHKRGKLTARERISLLLDPGSFVESDMFVEHRCADFGMAADKN
KFPGDSVVTGRGRINGRLVYVFSQQIIGWAQWLPLVISALWEAEDFTVFGGSLSGAHAQKICKIMDQAITVGAPVIGLNDSGGARIQEGVESLAGYADIF
LRNVTASGVIPQISLIMGPCAGGAVYSPALTDFTFMVKDTSYLFITGPDVVKSVTNEDVTQEELGGAKTHTTMSGVAHRAFENDVDALCNLRDFFNYLPL
SSQDPAPVRECHDPSDRLVPELDTIVPLESTKAYNMVDIIHSVVDEREFFEIMPNYAKNIIVGFARMNGRTVGIVGNQPKVASGCLDINSSVKGARFVRF
CDAFNIPLITFVDVPGFLPGTAQEYGGIIRHGAKLLYAFAEATVPKVTVITRKAYGGAYDVMSSKHLCGDTNYAWPTAEIAVMGAKGAVEIIFKGHENVE
AAQAEYIEKFANPFPAAVRGFVDDIIQPSSTRARICCDLDVLASKKVQRPWRKHANIPL
 
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